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USMLE Renal > Renal and Bladder Cancers, cysts and benign tumours + Renovascular disease > Flashcards

Renal and Bladder Cancers, cysts and benign tumours + Renovascular disease Flashcards

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USMLE® Step 1 flashcards
1
Q

What does autosomal dominant polycystic kidney disease present with?

A
  • Numerous cysts in cortex and medulla
  • Causes bilateral enlarged kidneys - ultimately destroying kidney parenchyma
  • Flank pain
  • Hematuria
  • Hypertension
  • UTI
  • Progressive renal failure in 50% of individuals
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2
Q

What mutations cause Aut Dom Polcystic Kidney Disease?

A
  • PKD1 (85% of cases) - chr 16

- PKD2 (15% of cases) - chr 4

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3
Q

What is Aut Dom Polycystic Kidney Disease associated with (other conditions)?

A
  • Berry aneurysms
  • Mitral valve prolapse
  • Benign hepatic cysts
  • Diverticulosis
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4
Q

What is Aut Dom Polycystic Kidney Disease treated with?

A

If hypertension or proteinuria develops treat with:

- ACEi or ARBs

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5
Q

Aut recessive polycystic kidney disease will have what on imaging?

A

Cystic dillation of CDs

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6
Q

Aut recessive polycystic kidney disease presents with?

A
  • In utero may cause Potter sequence
  • May present in infancy

After neonatal period:

  • Systemic hypertension
  • Progressive renal insufficiency
  • Portal hypertension from hepatic fibrosis
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7
Q

What does Autosomal Dominant tubulointerstitial kidney disease cause?

A
  • Causes tubulointerstitial fibrosis and progressive renal insufficiency w. inability to concentrate urine
  • Poor prognosis
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8
Q

What will Autosomal Dominant tubulointerstitial kidney disease look like?

A

Medullary cysts not visualised usually

- Smaller kidneys on US

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9
Q

What is the difference between simple and complex renal cysts?

A

Simple cysts
- Filled with ultrafiltrate (anechoic on US) - majority of renal masses and are typically asymptomatic

Complex renal cysts:
- May be septated, enhanced, or have solid components on imaging - require follow-up or removal due to possibility of RCC

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10
Q

What part of the nephron do RCC originate from?

A

PCT

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11
Q

What do RCC tumours look like?

A

Golden-yellow due to high lipid content (filled with lipid and carbohydrate)
- Polygonal clear cells

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12
Q

What do RCC cells spread?

A

Invade renal vein (may cause varicocele if L side)

  • > IVC
  • > Hematogenous spread
  • > Metastasize to lung and bone
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13
Q

What may RCC present with?

A
  • Hematuria
  • Palpable mass
  • Secondary polycythemia
  • Flank pain
  • Fever
  • Weight loss
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14
Q

How is RCC treated?

A

Surgery/ablation for localised disease

- Ipilimumab or targeted therapy for metastatic disease (rarely curative)

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15
Q

What is RCC resistant to?

A

Chemo and radiation therapy

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16
Q

What is the classic triad of RCC?

A
  • Flank pain
  • palpable mass
  • Hematuria
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17
Q

What is the most common primary renal malignancy?

A

RCC

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18
Q

What group is RCC most common in?

A

Men 50 - 70

- Increased risk in obese and smokers

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19
Q

What is RCC associated with?

A

Paraneoplastic syndromes PEAR

  • PTHrP
  • Ectopic EPO (polycythemia)
  • ACTH (Cushing)
  • Renin (Hypertension)
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20
Q

What is the most common subtype of RCC?

A

Clear cell

21
Q

What genes is RCC clear cell caused by?

A

Gene deletion on chr 3

- Sporadic or inherited as Von Hippel-Lindau syndrome

22
Q

What does Renal oncocytoma arise from?

A

Collecting ducts

23
Q

What kind of tumor is renal oncocytoma?

A

Benign

24
Q

What will renal oncocytoma reveal on histology?

A

Often resected to exclude malignancy

- Large eosinophilic cells with abundant mitochondria w/o perinuclear clearing (vs chromophobe in RCC)

25
Q

What does renal oncocytoma present with?

A
  • Painless hematuria
  • Flank pain
  • Abdo mass
    Same as RCC
26
Q

What is the most common malignancy of early childhood?

A

Nephroblastoma (ages 2 - 4)

27
Q

What is nephroblastoma also known as?

A

Wilms tumor

28
Q

What genes is nephroblastoma associated with?

A

Loss of function mutations of tumour suppressor genes

- WT1 or WT2 on chr 11

29
Q

What tissue does Wilms tumour / nephroblastoma contain?

A

Embryonic glomerular structures

30
Q

What may nephroblastoma present with?>

A
  • Large palpable unilateral flank mass
    Possibly:
  • hematuria
  • Hypertension
31
Q

What is urothelial carcinoma of the bladder also called?

A

Transitional cell carcinoma

32
Q

What is the most common tumour of the urinary tract system?

A

Transitional cell

- Urothelial carcinoma of bladder

33
Q

Where can transitional (urothelial carcinoma) of bladder occur?

A
  • Renal calyces
  • Renal pelvis
  • Ureters
  • Bladder
34
Q

What does urothelial carcinoma of bladder usually present with?

A

Painless hematuria (no casts)

35
Q

What is urothelial carcinoma of bladder caused by / associated with?

A

Pee SAC

  • Phenacetin (withdrawn pain-relief)
  • Smoking
  • Aromatic Amines (found in dyes)
  • Cyclophosphamide
36
Q

What squamous cell carcinoma of the bladder associated with (risk factors)?

A
  • Schistosoma haematobium
  • Chronic Cystitis
  • Smoking
  • Stones
37
Q

What does sq cell carcinoma of the bladder present with?

A

Painless hematuria (no casts)

38
Q

What sydromes are associated with nephroblastoma (wi;lms tumour)?

A
  • WAGR complex
  • Denys-Drash syndrome
  • Beckwith-Wiedemann syndrome
39
Q

What is WAGR complex/

A
  • Wilms tumor
  • Aniridia (absence of iris)
  • Genitourinary malfomrations
  • range of developmental delays
    WT1 deletion
40
Q

What is Denys-Drash syndrome?

A
  • Wilms tumor
  • Diffuse mesangial sclerosis (early onset nephrotic syndrome)
  • Dysgenesis of gonads (male pseudohermaphroditism)
    WT1 mutation
41
Q

What is Beckwith-Wiedemann syndrome?

A
  • Wilms tumour
  • Macroglossia
  • Organomegaly
  • Hemihyperplasia
  • Ompahalocele
    WT2 mutation
42
Q

What Wilms tumor associated syndromes are asociuated with WT1?

A
  • WAGR complex (WT1 deletion)

- Denys-Drash syndrome WT1 mutation

43
Q

What mutation is Beckwith-Wiedemann syndrome associated with?

A

WT2 mutation

44
Q

What is the most common cause of secondary hypertension in adults?

A

Renal artery stenosis

45
Q

What are the 2 main causes of RAS?

A
  • Atherosclerotic plaques (proximal 1/3) - older males/smokers
  • Fibromuscular dysplasia (distal 2/3s) - young females
46
Q

What will unilateral RAS show?

A

Affected kidney will atrophy (asymmetric kidney size)

- Renal venous sampling will show increased renin in affected kidney and decreased in unaffected

47
Q

What will happen to patients with bilateral RAS when started on a ACEi?

A

Sudden rise in creatinine

48
Q

What can RAS present with?

A
  • Severe/refractory HTN
  • Flash pulmonary edema
  • Epigastric/flank bruit
  • may have stenosis in other large vessels

USMLE Renal (11 decks)

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